title: Liposarcoma in children and young adults: a multi-institutional experience.
abstract: BACKGROUND
  There are limited data regarding the differences in clinical presentation and outcome of liposarcomas between adult and pediatric patients. The role of adjuvant radiotherapy in the treatment of childhood liposarcoma is unclear.
  
  
  METHODS
  A multi-institutional retrospective analysis of medical records was performed for patients ≤ 21 years of age presenting with a verified histologic diagnosis of liposarcoma.
  
  
  RESULTS
  Thirty-three patients were evaluable for this study, 23 of whom were male. Median age was 17.2 years. Twenty-four cases were myxoid subtype and 7 were pleomorphic subtype. In myxoid cases, 17 (71%) presented with extremity tumors; none had metastases. Eleven of these patients with myxoid subtype were treated with surgery only, seven with surgery + radiation, three with surgery + radiation + chemotherapy. Median radiation therapy dose for patients with myxoid tumors was 60 Gy. At median follow-up of 4.2 years (range 0.1-32.2 years), two patients relapsed with one death from progressive disease. In seven pleomorphic cases, four patients had primary tumors at central axial sites. Six patients (86%) received multimodal therapy, but six patients experienced relapse of disease. Four patients died from progressive disease.
  
  
  CONCLUSIONS
  Pediatric liposarcoma has a different spectrum of presentation compared to adult cases. Myxoid liposarcoma is the more common subtype, usually occurs in extremities, and has an excellent prognosis. Pleomorphic liposarcoma occurs in axial sites, and despite multimodal therapy, outcome is poor. Further study is needed to identify the optimal therapy for pediatric liposarcoma.
authors.affiliation: Division of Pediatrics, University of Texas MD Anderson Cancer Center, Houston, Texas 77030, USA. whuh@mdanderson.org
authors.forename: Winston W
authors.initials: WW
authors.lastname: Huh
authors.forename: Carrie
authors.initials: C
authors.lastname: Yuen
authors.forename: Mark
authors.initials: M
authors.lastname: Munsell
authors.forename: Andrea
authors.initials: A
authors.lastname: Hayes-Jordan
authors.forename: Alexander J
authors.initials: AJ
authors.lastname: Lazar
authors.forename: Shreyaskumar
authors.initials: S
authors.lastname: Patel
authors.forename: Wei-Lien
authors.initials: WL
authors.lastname: Wang
authors.forename: Nadia
authors.initials: N
authors.lastname: Barahmani
authors.forename: M Fatih
authors.initials: MF
authors.lastname: Okcu
authors.forename: John
authors.initials: J
authors.lastname: Hicks
authors.forename: Larisa
authors.initials: L
authors.lastname: Debelenko
authors.forename: Sheri L
authors.initials: SL
authors.lastname: Spunt
mesh_terms: D000293:Adolescent; D000970:Antineoplastic Agents / Q000627:therapeutic use; D002648:Child; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008080:Liposarcoma / Q000401:mortality* / Q000473:pathology* / Q000628:therapy; D008297:Male; D011878:Radiotherapy; D012189:Retrospective Studies; D012983:Soft Tissue Neoplasms / Q000401:mortality / Q000473:pathology* / Q000628:therapy*; D055815:Young Adult
chemical_list: D000970:Antineoplastic Agents